The Korean Journal of Medicine

The Korean Association of Internal Medicine (대한내과학회)
권호 표지

A Case of Pulmonary Lymphangioleiomyomatosis Associated with Tuberous Sclerosis and Renal Angiomyolipoma

신장을 침범한 결정성 경화증에서 발생한 폐의 임파관평활근종증 1예

  • Park, So-Hee (Department of Pulmonology and Critical Care, Kyung Hee University East-West Neo Medical Center) ;
  • Son, Ju-Woong (Department of Internal Medicine and Pulmonology and Critical Care, Kyung Hee University School of Medicine) ;
  • Park, Choul-Ki (Department of Internal Medicine and Pulmonology and Critical Care, Kyung Hee University School of Medicine) ;
  • Park, Myung-Jae (Department of Internal Medicine and Pulmonology and Critical Care, Kyung Hee University School of Medicine) ;
  • Yoo, Jee-Hong (Department of Pulmonology and Critical Care, Kyung Hee University East-West Neo Medical Center) ;
  • Kang, Hong-Mo (Department of Internal Medicine and Pulmonology and Critical Care, Kyung Hee University School of Medicine)
  • 박소희 (경희대학교 의과대학 강동경희대학교병원 호흡기내과) ;
  • 손주웅 (경희대학교 의과대학 내과학교실) ;
  • 박철기 (경희대학교 의과대학 내과학교실) ;
  • 박명재 (경희대학교 의과대학 내과학교실) ;
  • 유지홍 (경희대학교 의과대학 강동경희대학교병원 호흡기내과) ;
  • 강홍모 (경희대학교 의과대학 내과학교실)
  • Published : 2011.12.01
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Abstract

Tuberous sclerosis is an autosomal dominant disorder characterized by facial skin lesions, epilepsy, and mental retardation. Pulmonary involvement in tuberous sclerosis is rare and shows characteristic reticulonodular infiltration and cystic changes. Lymphangioleiomyomatosis is characterized by the progressive proliferation of smooth muscle cells and occurs in 0.1-1% of patients with tuberous sclerosis. We encountered a case of pulmonary lymphangioleiomyomatosis associated with tuberous sclerosis and bilateral renal angiomyolipoma in a 31-year-old female patient. This case is reported here along with a brief review of the literature.

저자들은 신장의 혈관근 지방종과 간질로 결절성 경화증을 진단 받고 추적관찰 중인 환자에서 발생한 점진적인 호흡곤란으로 시행한 흉부 전산화 단층 촬영과 병리 조직 소견에서 HMB-45 양성의 폐 림프관 혈관종증을 진단한 1예를 경험하였기에 문헌과 함께 보고하는 바이다.

Keywords

References

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